ObjectiveTo summarize the diagnosis and clinicopathological features of primary intestinal extranodal nasal type NK/T-cell lymphoma (PI-ENKTCL), and to enhance the diagnostic and differential diagnosis of this rare disease.MethodsThe clinicopathologic data of a patient with PI-ENKTCL admitted to Gansu Provincial Hospital in January 2019 were retrospectively analyzed.ResultsWe diagnosed PI-ENKTCL doubtfully by PET-CT , and after the discussion of MDT, we finished radical operation which achieved R0 resection. The pathological type was consistent with PI-ENKTCL diagnosis. The operation lasted 280 minutes, and the intraoperative blood loss was about 50 mL. Postoperative pathology confirmed that the tumor margin was negative and no postoperative complication occurred, and the patient was discharged successfully on the 13th day after the operation. The patient, followed for five months after operation, was treated with L-GMOEX [gemcitabine+oxaliplatin combined with L-asparaginase (L-ASP)] chemotherapy for four periods at time, and the treatment effect continued to be followed up.ConclusionsIt is necessary to discuss by MDT for doubtfully diagnosing PI-ENKTCL because of the disease being rare. The imaging diagnosis is mainly PET-CT. Surgical resection is the main treatment, we should formulate the best chemotherapy according to post-operation pathological results, for improving the patient’s prognosis and prolonging patient’s life with PI-ENKTCL.